Spontaneous rupture of abdominal aorta — an unusual manifestation of an idiopathic cystic medial necrosis (Gsell-Erdheim syndrome) and literature overview

Abstract

In this report, a case of a male, 40-year-old patient who suffered from spontaneous rupture of undilated abdominal aorta, a manifestation of idiopathic cystic media necrosis (Gsell-Erdheim syndrome), is presented. Spontaneous ruptures of medium-to-large arteries observed in this syndrome are a consequence of elastic fibres degeneration and glucose-amino-glycans (GAG) depositions in the arterial media. Gsell-Erdheim syndrome is also observed in a large